Using Telerehabilitation to Deliver a Home Exercise Program to Youth With Arthrogryposis: Single Cohort Pilot Study.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is characterized by joint contractures and muscle weakness, which limit daily activities. Youths with AMC require frequent physical therapeutic follow-ups to limit the recurrence of contractures and maintain range of motion (ROM) and muscle strength; however, access to specialized care may be limited because of geographical distance. Telerehabilitation can offer a potential solution for delivering frequent follow-ups for youth with AMC, but research on the use of telerehabilitation in children with musculoskeletal disorders is scarce. OBJECTIVE: The study aims to evaluate the feasibility of delivering a home exercise program (HEP) by using telerehabilitation for youth with AMC. We also aim to explore the effectiveness of the HEP as a secondary aim. METHODS: Youths aged between 8 and 21 years with AMC were recruited at the Shriners Hospitals for Children-Canada. The participants completed baseline and post-HEP questionnaires (the Physical Activity Questionnaire for Adolescents, Pediatrics Outcomes Data Collection Instrument, and Adolescent and Pediatric Pain Tool), and clinicians assessed their active ROM using a virtual goniometer. Clinicians used the Goal Attainment Scale with the participants to identify individualized goals to develop a 12-week HEP and assess the achievement of these goals. Follow-ups were conducted every 3 weeks to adjust the HEP. Data on withdrawal rates and compliance to the HEP and follow-ups were collected to assess the feasibility of this approach. The interrater reliability of using a virtual goniometer was assessed using the intraclass correlation coefficient and associated 95% CI. Nonparametric tests were used to evaluate feasibility and explore the effectiveness of the HEP. RESULTS: Of the 11 youths who were recruited, 7 (median age: 16.9 years) completed the HEP. Of the 47 appointments scheduled, 5 had to be rescheduled in ≤24 hours. The participants performed their HEP 2.04 times per week (95% CI 1.25-4.08) and reported good satisfaction with the approach. A general intraclass correlation coefficient of 0.985 (95% CI 0.980-0.989) was found for the web-based ROM measurement. Individualized goals were related to pain management; endurance in writing, standing, or walking; sports; and daily activities. In total, 12 of the 15 goals set with the participants were achieved. Statistically significant improvements were observed in the pain and comfort domain of the Pediatrics Outcomes Data Collection Instrument (preintervention: median 71; 95% CI 34-100; postintervention: median 85; 95% CI 49-100; P=.08) and Physical Activity Questionnaire for Adolescents (preintervention: median 1.62; 95% CI 1.00-2.82; postintervention: median 2.32; 95% CI 1.00-3.45; P=.046). CONCLUSIONS: The remote delivery of an HEP for youth with AMC is feasible. Promising results were found for the effectiveness of the HEP in helping youths with AMC to achieve their goals. The next step will be to assess the effectiveness of this exercise intervention in a randomized controlled trial. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): RR2-10.2196/18688.

Inter-rater Reliability of the McKenzie Method of Mechanical Diagnosis and Therapy for the Provisional Classification of Low Back Pain in Adolescents and Young Adults.

OBJECTIVE: To investigate the inter-rater reliability of Mechanical Diagnosis and Therapy (MDT)-trained Diplomats in classifying adolescents and young adults with lumbar pain. METHODS: Forty-three participants (mean age 15 ± 2 years) with lumbar pain, with or without lower extremity symptoms, were assessed simultaneously by three MDT Diploma holders and classified into one of three groups: 1) Derangement, 2) Dysfunction, 3) Postural/OTHER. Inter-rater reliability was calculated using the Fleiss kappa statistics with 95% confidence intervals (CI). Analyses were repeated with the younger (11 to 15 years old) and older (16 to 21 years old) age groups. RESULTS: There was moderate reliability (Fleiss kappa = 0.50, 95% CI = 0.45 to 0.54) for the entire sample, which was statistically significant (p < 0.001). There was good reliability in older participants (Fleiss kappa = 0.63, 95% CI = 0.57 to 0.70), but poor reliability in younger participants (Fleiss kappa = 0.33, 95% CI = 0.27 to 0.39). There was 100% agreement in classifications among assessors for 70% of participants. DISCUSSION: The MDT system has moderate reliability when classifying lumbar pain in adolescents and young adults. Future reliability studies may include a balanced group for classifications or a second session.

A Telerehabilitation Intervention for Youths With Arthrogryposis Multiplex Congenita: Protocol for a Pilot Study.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is characterized by joint contractures present in at least two body areas. In addition to these contractures, individuals with AMC can have decreased muscle mass, leading to limitations in activities of daily living. Exercise has the potential to maintain or improve the range of motion and muscle strength. However, this type of intervention necessitates frequent follow ups that are currently difficult to provide for youths with AMC because they often live far from a specialized hospital. To overcome this distance challenge, telecommunication technologies can be used to deliver rehabilitation remotely, which is called telerehabilitation. The study protocol for one such type of rehabilitation will be presented in this paper. OBJECTIVE: This pilot study aims to (1) evaluate the feasibility of using telerehabilitation to provide a home exercise program for youths with AMC, and (2) assess the effectiveness of a home exercise program. METHODS: A total of 10 youths aged 8-21 years with AMC will be recruited. The intervention consists of a 12-week individualized home-based exercise program delivered remotely using telerehabilitation. At baseline, youths will complete the Physical Activity Questionnaire for Adolescents and the Pediatrics Outcomes Data Collection Instrument to assess pain, function, and level of physical activity. During the first telerehabilitation meeting, the rehabilitation therapists will measure range of motion using a virtual goniometer and assess the youth's functional level. The therapists will then use the Goal Attainment Scale to set objectives and develop the individualized intervention. Follow ups will occur every 3 weeks to make sure exercises are performed safely and to progress the exercises when needed. At the end of the 12-week intervention, rehabilitation therapists will re-evaluate the youth using the same outcome measures as the initial evaluation. The youths will be asked to complete the same questionnaires, with the addition of questions about their satisfaction regarding the intervention. Nonparametric and descriptive statistics will be used to evaluate the feasibility and effectiveness. RESULTS: Ethics approval was obtained in October 2018. Recruitment and data collection started in January 2019 and was completed in May 2020. CONCLUSIONS: This pilot study will help us learn how a large-scale project may work in practice to improve outcomes in physical activity, pain, and function, and goal attainment among youths with AMC, thus informing a future clinical trial. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/18688.

Selective Dorsal Root Rhizotomy for Spastic Cerebral Palsy: A Longitudinal Case-Control Analysis of Functional Outcome.

BACKGROUND: The benefit of selective dorsal rhizotomies (SDR) on motor function relative to the cerebral palsy (CP) natural history remains unknown. OBJECTIVE: To determine the functional benefit of SDR over the longitudinal CP natural history. METHODS: Retrospective, single-center, case-control study of patients post-SDR after 1990. Inclusion criteria were the following: diagnosis of spastic CP, at least 1 preoperative and 1 postoperative Gross Motor Function Measure (GMFM-88), at least 1 yr of postoperative follow-up. GMFM-88 assessments were performed at 1, 2, 3, 5, 10, and 15 yr postoperatively and converted to GMFM-66. Cases were stratified by preoperative Gross Motor Function Classification System (GMFCS) and matched against their expected natural history using published reference centiles. After age 12, our cohort and matched controls were also fitted to published nonlinear mixed models of GMFM-66 evolution over time. RESULTS: Analysis included 190 patients. Median follow-up, 5.3 yr (range: 1-16.9), median age at surgery, 4.6 yr, and 81.6% of patients grouped as GMFCS II or III pre-op. SDR patients performed statistically significantly better than their expected natural history (P < .0005). At 21 yr old, a modeled benefit of 8.435 was observed for GMFCS I (P = .0051), 0.05 for GMFCS II (P = .9647), 6.31 for GMFCS III (P < .0001), and 1.191 for GMFCS IV patients (P = .0207). CONCLUSION: These results support the use of SDR in carefully selected spastic CP children.

Functional Gains in Children With Spastic Hemiplegia Following a Tendon Achilles Lengthening Using Computerized Adaptive Testing-A Pilot Study.

PURPOSE: This pilot study evaluated the outcomes of tendon Achilles lengthening in 12 children (mean age: 11.2 years) with spastic hemiplegia. METHODS: Cerebral Palsy Computer Adaptive Tests, the timed up-and-go, the Gross Motor Function Measure, the Gillette Functional Assessment Questionnaire, and the Pediatric Outcomes Data Collection Instrument were administered at baseline and at 6, 12, and 24 months postsurgery. RESULTS: Significant improvement at the latest follow-up (12-24 months following surgery) was seen in all domains of the Cerebral Palsy Computer Adaptive Test: activity (P = .017), lower extremity (P = .005), global (P = .005), pain (P = .005), and fatigue (P = .028), as well as in the Gross Motor Function Measure-D domain (P = .02) and the mobility domain of the Pediatric Outcomes Data Collection Instrument (P = .04). CONCLUSION: These findings indicate that the tendon Achilles lengthening improved functional outcome in these children as measured by tests of physical function, walking speed, and activity performance.

Long-term functional benefits of selective dorsal rhizotomy for spastic cerebral palsy.

OBJECT: Large-scale natural history studies of gross motor development have shown that children with spastic cerebral palsy (CP) plateau during childhood and actually decline through adolescence. Selective dorsal rhizotomy (SDR) is a well-recognized treatment for spastic CP, but little is known about long-term outcomes of this treatment. The purpose of this study was to assess the durability of functional outcomes in a large number of patients through adolescence and into early adulthood using standardized assessment tools. METHODS: The authors analyzed long-term follow-up data in children who had been evaluated by a multidisciplinary team preoperatively and at 1, 5, 10, and 15 years after SDR. These evaluations included quantitative, standardized assessments of lower-limb tone (Ashworth Scale), Gross Motor Function Measure (GMFM), and performance of activities of daily living (ADLs) by the Pediatric Evaluation of Disability Inventory in children who had been stratified by motor severity using the Gross Motor Function Classification System (GMFCS). In addition, group-based trajectory modeling (GBTM) was used to identify any heterogeneity of response to SDR among these treated children, and to find which pretreatment variables might be associated with this heterogeneity. Finally, a chart review of adjunct orthopedic procedures required by these children following SDR was performed. RESULTS: Of 102 patients who underwent preoperative evaluations, 97, 62, 57, and 14 patients completed postoperative assessments at 1, 5, 10, and 15 years, respectively. After SDR, through adolescence and into early adulthood, statistically significant durable improvements in lower-limb muscle tone, gross motor function, and performance of ADLs were found. When stratified by the GMFCS, long-lasting improvements for GMFCS Groups I, II, and III were found. The GBTM revealed 4 groups of patients who responded differently to SDR. This group assignment was associated with distribution of spasticity (diplegia was associated with better outcomes than triplegia or quadriplegia) and degree of hip adductor spasticity (Ashworth score < 3 was associated with better outcomes than a score of 3), but not with age, sex, degree of ankle plantar flexion spasticity, or degree of hamstring spasticity. In a sample of 88 patients who had complete records of orthopedic procedures and botulinum toxin (Botox) injections, 52 (59.1%) underwent SDR alone, 11 (12.5%) received only Botox injections in addition to SDR, while 25 patients (28.4%) needed further lower-extremity orthopedic surgery after SDR. CONCLUSIONS: In the majority of patients, the benefits of SDR are durable through adolescence and into early adulthood. These benefits include improved muscle tone, gross motor function, and performance of ADLs, as well as a decreased need for adjunct orthopedic procedures or Botox injections. The children most likely to display these long-term benefits are those in GMFCS Groups I, II, and III, with spastic diplegia, less hip adductor spasticity, and preoperative GMFM scores greater than 60.

Determinants of responsiveness to botulinum toxin, casting, and bracing in the treatment of spastic equinus in children with cerebral palsy.

AIM: The objective was to determine whether specific intrinsic (age, pattern of cerebral palsy [CP], child's motivation) and extrinsic (number of treatments, parenting stress) characteristics were associated with responsiveness to botulinum toxin A (BoNT-A) injections in children with CP 3 months after injection into the gastrocnemius muscle. METHOD: Children with hemiplegia or diplegia recruited from a BoNT-A programme were evaluated before and 3 months following injection of BoNT-A into the gastrocnemius. Outcome measures included muscle tone, range of motion, gait pattern, level of ambulation, gross motor function, and functional independence. Determinants of responsiveness to BoNT-A considered were age, number of treatments, distribution of CP, parenting stress, and motivation. RESULTS: Thirty-one children were recruited (17 males, 14 females)--22 with hemiplegia and nine with diplegia. Twenty-eight were classified at Gross Motor Function Classification System (GMFCS) level I and three at level III. The mean age was 6 years 4 months (SD 2y 11mo). Younger age (p=0.015) and fewer number (p=0.024) of BoNT-A treatments were associated with greater change in gross motor function. Child's motivation and parenting stress were significantly associated with improvements in muscle tone (p=0.006-0.017), passive range of motion (p=0.008-0.033), gait pattern (p=0.005-0.042), level of ambulation (p=0.001-0.043), and functional independence (p=0.004-0.027). INTERPRETATION: The results indicate that child, family, and treatment characteristics influence the degree of responsiveness to BoNT-A treatment. The contribution of contextual factors (personal and environmental) on responsiveness may be underappreciated in children with CP.